Ebsteins anomaly of the tricuspid valve pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] and Claudia P. Hochberg, M.D. [2]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [3]}; Keri Shafer, M.D. [4] Priyamvada Singh, MBBS [[5]] Assistant Editor-In-Chief: Kristin Feeney, B.S. [[6]]

Pathophysiology

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The pathophysiology of Ebstein's anomaly depends on the morphology of tricuspid valve and right ventricle.

Tricuspid valve

• The anterior leaflet of the tricuspid valve is malformed in this condition i.e. they are attached to tricuspid valve annulus or to the right ventricular endocardium
• The septal and posterior leaflets are absent most of the times.
• Due to these changes the tricuspid valve may becomes funnel-shaped and incompetent.

Right ventricle

The right ventricular changes are secondary to the malformed tricuspid valves. The right ventricle can thus be divided into two parts by the valve

• The downward extension of the tricuspid valve causes 'atrialization' of the proximal part of the right ventricle
• The small distal part, the right ventricle proper, thus is reduced in size and sometimes comprises only of the right outflow tract.

• 50% of patients have ASD or patent foramen ovale
• 25% have accesory pathway